Skip to main content

Advertisement

SpringerLink
Log in

Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases

  • Review
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Objectives

The objectives of this study were to analyse the literature on Sweet’s syndrome in childhood focussing on associated diseases and to suggest possible screening procedures for this group of patients. Furthermore, two new patients with Sweet’s syndrome are reported.

Methods

A literature search was performed on Pub med using search terms “sweet* syndrome*” and neutrophil* dermatos*. Patients were subdivided into the following groups: classic/idiopathic, paraneoplastic, and parainflammatory Sweet’s syndrome.

Results

The literature search revealed 64 patients (including our two patients) who were diagnosed with Sweet’s syndrome in childhood and adolescence; 27 (42%) patients were categorized as “classic/idiopathic Sweet’s syndrome”. In 37 patients (58%) chronic associated diseases were reported. Out of these, 21 (33%) patients were categorized as “parainflammatory Sweet’s syndrome” including chronic recurrent multifocal osteomyelitis, vasculitis with aortitis, recurrent infections due to immunodeficiencies, arthritis, and systemic lupus erythematosus. Sixteen (25%) patients were categorized as “paraneoplastic Sweet’s syndrome” comprising both malignant and premalignant diseases like leukemia, aplastic anaemia, and Fanconi anaemia. As all five (8%) patients treated with drugs (granulocyte-colony stimulating factor, retinoid acid) suffered from malignant, premalignant, or parainflammatory diseases, these patients were categorized according to the underlying disease. Two new children with Sweet’s syndrome and associated diseases are presented here, one of them suffering from recurrent infections and trisomy 21, while the other was diagnosed with CNS vasculitis 5 1/2 years after the primary diagnosis.

Conclusions

Sweet’s syndrome should be considered in differential diagnosis of prolonged fever with cutaneous involvement. As most cases of pediatric Sweet’s syndrome are associated with other diseases we suggest careful screening and monitoring of these patients especially concerning malignant/premalignant diseases, immunodeficiencies, cardiovascular involvement, autoimmune diseases, and drug associations.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

Abbreviations

AA:

Aplastic anemia

AML:

Acute myelogenous leukemia

ALL:

Acute lymphoblastic leukemia

ATRA:

All-trans retinoic acid

BMT:

Bone marrow transplantation

CDA:

Congenital dyserythropoeitic anemia

CGD:

Chronic Granulomatous disease

CRMO:

Chronic recurrent multifocal osteomyelitis

FA:

Fanconi anemia

G-CSF:

Granulocyte colony stimulating factor

HIV:

Human immunodeficiency virus infection

jcML:

Juvenile chronic myelogenous leukemia

MBL:

Medulloblastoma

MDS:

Myelodysplastic syndrome

NBL:

Neuroblastoma

SIRS:

Systemic inflammatory response syndrome

References

  1. Al-Saad K, Khanani MF, Naqvi A, Krafchik B, Grant R, Pappo A (2004) Sweet syndrome developing during treatment with all-trans retinoic acid in a child with acute myelogenous leukemia. J Pediatr Hematol Oncol 26:197–199. doi:10.1097/00043426-200403000-00011

    Article  PubMed  Google Scholar 

  2. Astudillo L, Loche F, Reynish W, Rigal-Huguet F, Lamant L, Pris J (2002) Sweet’s syndrome associated with retinoic acid syndrome in a patient with promyelocytic leukemia. Ann Hematol 81:111–114. doi:10.1007/s00277-001-0416-5

    Article  PubMed  CAS  Google Scholar 

  3. Attili RV, Hira SK, Dube MK (1983) Sweet’s syndrome in a child (a case report). Med J Zambia 17:92–93

    PubMed  CAS  Google Scholar 

  4. Bajwa RP, Marwaha RK, Garewal G, Rajagopalan M (1993) Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in myelodysplastic syndrome. Pediatr Hematol Oncol 10:343–346. doi:10.3109/08880019309029513

    Article  PubMed  CAS  Google Scholar 

  5. Baron F, Sybert VP, Andrews RG (1989) Cutaneous and extracutaneous neutrophilic infiltrates (Sweet syndrome) in three patients with Fanconi anemia. J Pediatr 115:726–729. doi:10.1016/S0022-3476(89)80649-3

    Article  PubMed  CAS  Google Scholar 

  6. Boatman BW, Taylor RC, Klein LE, Cohen BA (1994) Sweet’s syndrome in children. South Med J 87:193–196. doi:10.1097/00007611-199402000-00009

    Article  PubMed  CAS  Google Scholar 

  7. Brady RC, Morris J, Connelly BL, Boiko S (1999) Sweet’s syndrome as an initial manifestation of pediatric human immunodeficiency virus infection. Pediatrics 104:1142–1144. doi:10.1542/peds.104.5.1142

    Article  PubMed  CAS  Google Scholar 

  8. Burnham JM, Cron RQ (2005) Sweet syndrome as an initial presentation in a child with systemic lupus erythematosus. Lupus 14:974–975. doi:10.1191/0961203305lu2236xx

    Article  PubMed  CAS  Google Scholar 

  9. Callen JP (2002) Neutrophilic dermatoses. Dermatol Clin 20:409–419. doi:10.1016/S0733-8635(02)00006-2

    Article  PubMed  Google Scholar 

  10. Campos LM, Castellanos AL, Afiune JY, Kiss MH, Silva CA (2005) Takayasu’s arteritis with aortic aneurysm associated with Sweet’s syndrome in childhood. Ann Rheum Dis 64:168–169. doi:10.1136/ard.2003.006353

    Article  PubMed  CAS  Google Scholar 

  11. Christensen CC, Gonzalez-Crussi F (1983) Postinflammatory elastolysis and cutis laxa: report of a case with aortitis. Pediatr Pathol 1:199–210. doi:10.3109/15513818309040658

    Article  PubMed  CAS  Google Scholar 

  12. Collins P, Rogers S, Keenan P, McCabe M (1991) Acute febrile neutrophilic dermatosis in childhood (Sweet’s syndrome). Br J Dermatol 124:203–206. doi:10.1111/j.1365-2133.1991.tb00435.x

    Article  PubMed  CAS  Google Scholar 

  13. Dunn TR, Saperstein HW, Biederman A, Kaplan RP (1992) Sweet syndrome in a neonate with aseptic meningitis. Pediatr Dermatol 9:288–292. doi:10.1111/j.1525-1470.1992.tb00350.x

    Article  PubMed  CAS  Google Scholar 

  14. Edwards TC, Stapleton FB, Bond MJ, Barrett FF (1986) Sweet’s syndrome with multifocal sterile osteomyelitis. Am J Dis Child 140:817–818

    PubMed  CAS  Google Scholar 

  15. Eghrari-Sabet JS, Hartley AH (1994) Sweet’s syndrome: an immunologically mediated skin disease. Ann Allergy 72:125–128

    PubMed  CAS  Google Scholar 

  16. Elliott SP, Mallory SB (1999) Sweet syndrome: an unusual presentation of chronic granulomatous disease in a child. Pediatr Infect Dis J 18:568–570. doi:10.1097/00006454-199906000-00025

    Article  PubMed  CAS  Google Scholar 

  17. Garewal G, Marwaha RK, Ray R, Marwaha N (1993) Clinico-hematological profile and natural history of childhood myelodysplastic syndromes. Indian J Pediatr 60:573–581. doi:10.1007/BF02751436

    Article  PubMed  CAS  Google Scholar 

  18. Garty BZ, Levy I, Nitzan M, Barak Y (1996) Sweet syndrome associated with G-CSF treatment in a child with glycogen storage disease type Ib. Pediatrics 97:401–403

    PubMed  CAS  Google Scholar 

  19. Goh CL, Alora M, Kohar Y (1996) Sweet’s syndrome in a skin clinic in Singapore: an epidemiology study. Ann Acad Med Singapore 25:222–227

    PubMed  CAS  Google Scholar 

  20. Gray LC, Abele DC (1998) Annular erythematous plaques and tibial pain in a child. Sweet syndrome. Arch Dermatol 134:625–626, 628–629. doi:10.1001/archderm.134.5.625-a

    Google Scholar 

  21. Guia JM, Frias J, Castro FJ, Gracian M (1999) Cardiovascular involvement in a boy with Sweet’s syndrome. Pediatr Cardiol 20:295–297. doi:10.1007/s002469900468

    Article  PubMed  CAS  Google Scholar 

  22. Gyorfy A, Kovacs T, Szegedi I, Olah E, Kiss C (2003) Sweet syndrome associated with 13-cis-retinoic acid (isotretinoin) therapy. Med Pediatr Oncol 40:135–136. doi:10.1002/mpo.10089

    Article  PubMed  Google Scholar 

  23. Haliasos E, Soder B, Rubenstein DS, Henderson W, Morrell DS (2005) Pediatric Sweet syndrome and immunodeficiency successfully treated with intravenous immunoglobulin. Pediatr Dermatol 22:530–535. doi:10.1111/j.1525-1470.2005.00133.x

    Article  PubMed  Google Scholar 

  24. Hassouna L, Nabulsi-Khalil M, Mroueh SM, Zaynoun ST, Kibbi AG (1996) Multiple erythematous tender papules and nodules in an 11-month-old boy. Sweet syndrome (SS) (acute febrile neutrophilic dermatosis). Arch Dermatol 132:1507–1510

    Google Scholar 

  25. Havaldar PV, Kumar H, Koppad AM, Bellad R, Mogale KD (1998) Acute febrile neutrophilic dermatosis (Sweet’s syndrome). Indian Pediatr 35:1017–1020

    PubMed  CAS  Google Scholar 

  26. Hazen PG, Kark EC, Davis BR, Carney JF, Kurczynski E (1983) Acute febrile neutrophilic dermatosis in children. Report of two cases in male infants. Arch Dermatol 119:998–1002. doi:10.1001/archderm.119.12.998

    Article  PubMed  CAS  Google Scholar 

  27. Herron MD, Coffin CM, Vanderhooft SL (2005) Sweet syndrome in two children. Pediatr Dermatol 22:525–529. doi:10.1111/j.1525-1470.2005.00132.x

    Article  PubMed  Google Scholar 

  28. Heyl TSIW, Cronje RE (1971) Post-inflammatory cutis laxa and aortitis (acquired systemic elastolysis). Br J Dermatol 85:37. doi:10.1111/j.1365-2133.1971.tb14107.x

    Article  Google Scholar 

  29. Itami S, Nishioka K (1980) Sweet’s syndrome in infancy. Br J Dermatol 103:449–451. doi:10.1111/j.1365-2133.1980.tb07270.x

    Article  PubMed  CAS  Google Scholar 

  30. Jain KK (1996) Sweet’s syndrome associated with granulocyte colony-stimulating factor. Cutis 57:107–110

    PubMed  CAS  Google Scholar 

  31. Keller J, Spira I (1986) Sweet syndrome with immune complex vasculitis in a child. Z Hautkr 61:1351–1360

    Google Scholar 

  32. Kibbi AG, Zaynoun ST, Kurban AK, Najjar SS (1985) Acute febrile neutrophilic dermatosis (Sweet’s syndrome): case report and review of the literature. Pediatr Dermatol 3:40–44. doi:10.1111/j.1525-1470.1985.tb00484.x

    Article  PubMed  CAS  Google Scholar 

  33. Klock JC, Oken RL (1976) Febrile neutrophilic dermatosis in acute myelogenous leukemia. Cancer 37:922–927. doi:10.1002/1097-0142(197602)37:2<922::AID-CNCR2820370245>3.0.CO;2-P

    Article  PubMed  CAS  Google Scholar 

  34. Kourtis AP (2002) Sweet syndrome in infants. Clin Pediatr (Phila) 41:175–177. doi:10.1177/000992280204100308

    Google Scholar 

  35. Krilov LR, Jacobson M, Shende A (1987) Acute febrile neutrophilic dermatosis (Sweet’s syndrome) presenting as facial cellulitis in a child with juvenile chronic myelogenous leukemia. Pediatr Infect Dis J 6:77–79. doi:10.1097/00006454-198701000-00025

    Article  PubMed  CAS  Google Scholar 

  36. Laissaoui K, Amal S, Hocar O, Benadra F, Alatawna H, Belaabidia B (2005) Sweet’s syndrome in a child. Ann Dermatol Venereol 132:32–34. doi:10.1016/S0151-9638(05)79192-4

    Google Scholar 

  37. Larsson LG, Baum J (1985) Acute febrile neutrophilic dermatosis (Sweet’s syndrome). Successful treatment with short term corticosteroids. J Rheumatol 12:1000–1003

    PubMed  CAS  Google Scholar 

  38. Lear JT, Atherton MT, Byrne JP (1997) Neutrophilic dermatoses: pyoderma gangrenosum and Sweet’s syndrome. Postgrad Med J 73:65–68

    PubMed  CAS  Google Scholar 

  39. Levin DL, Esterly NB, Herman JJ, Boxall LB (1981) The Sweet syndrome in children. J Pediatr 99:73–78. doi:10.1016/S0022-3476(81)80960-2

    Article  PubMed  CAS  Google Scholar 

  40. Lipp KE, Shenefelt PD, Nelson RP Jr, Messina JL, Fenske NA (1999) Persistent Sweet’s syndrome occurring in a child with a primary immunodeficiency. J Am Acad Dermatol 40:838–841. doi:10.1053/jd.1999.v40.a95650

    Article  PubMed  CAS  Google Scholar 

  41. Litt R, Branski D, Gross-Kieselstein E, Abrahamov A, Rubinow A (1986) Sweet syndrome in early childhood. Eur J Pediatr 145:303–305. doi:10.1007/BF00439405

    Article  PubMed  CAS  Google Scholar 

  42. Loeys BL, Schwarze U, Holm T, Callewaert BL, Thomas GH, Pannu H et al (2006) Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med 355:788–798. doi:10.1056/NEJMoa055695

    Article  PubMed  CAS  Google Scholar 

  43. Majeed HA, Kalaawi M, Mohanty D, Teebi AS, Tunjekar MF, al-Gharbawy F et al (1989) Congenital dyserythropoietic anemia and chronic recurrent multifocal osteomyelitis in three related children and the association with Sweet syndrome in two siblings. J Pediatr 115:730–734. doi:10.1016/S0022-3476(89)80650-X

    Article  PubMed  CAS  Google Scholar 

  44. Maleville J, Taieb A (1993) Acute febrile neutrophilic dermatosis in childhood (Sweet syndrome). Pediatr Dermatol 10:298–299. doi:10.1111/j.1525-1470.1993.tb00386.x

    Article  PubMed  CAS  Google Scholar 

  45. Malone JC, Slone SP, Wills-Frank LA, Fearneyhough PK, Lear SC, Goldsmith LJ et al (2002) Vascular inflammation (vasculitis) in sweet syndrome: a clinicopathologic study of 28 biopsy specimens from 21 patients. Arch Dermatol 138:345–349. doi:10.1001/archderm.138.3.345

    Article  PubMed  Google Scholar 

  46. McDermott MB, Corbally MT, O ’Marcaigh AS (2001) Extracutaneous Sweet syndrome involving the gastrointestinal tract in a patient with Fanconi anemia. J Pediatr Hematol Oncol 23:59–62. doi:10.1097/00043426-200101000-00015

    Article  PubMed  CAS  Google Scholar 

  47. Munoz Hoyos A, Loscertales Abril M, Caballero MT, Hernandez Gomez MV, Delgado V, Ruiz Extremera A et al (1983) Sweet’s syndrome in an infant. Pediatrie 38:253–259

    Google Scholar 

  48. Muster AJ, Bharati S, Herman JJ, Esterly NB, Gonzales-Crussi F, Holbrook KA (1983) Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis. J Pediatr 102:243–248. doi:10.1016/S0022-3476(83)80529-0

    Article  PubMed  CAS  Google Scholar 

  49. Nurre LD, Rabalais GP, Callen JP (1999) Neutrophilic dermatosis-associated sterile chronic multifocal osteomyelitis in pediatric patients: case report and review. Pediatr Dermatol 16:214–216. doi:10.1046/j.1525-1470.1999.00058.x

    Article  PubMed  CAS  Google Scholar 

  50. Ohga S, Nomura A, Takada H, Terao H, Harada N, Hara T (2002) Expansion of trisomy 8 and Sweet syndrome in a prolonged course of aplastic anemia. J Pediatr Hematol Oncol 24:64–68. doi:10.1097/00043426-200201000-00017

    Article  PubMed  Google Scholar 

  51. Oshitari K, Hida T, Takahashi M, Okada AA, Shiobara T (2004) Sweet syndrome presenting as orbital cellulitis. Nippon Ganka Gakkai Zasshi 108:162–165

    Google Scholar 

  52. Otheo E, Ros P, Vazquez JL, Carrillo R, Moreno R, Maldonado S et al (2002) Systemic inflammatory response syndrome associated with Sweet’s syndrome. Pediatr Crit Care Med 3:190–193. doi:10.1097/00130478-200204000-00020

    Article  PubMed  Google Scholar 

  53. Parsapour K, Reep MD, Gohar K, Shah V, Church A, Shwayder TA (2003) Familial Sweet’s syndrome in 2 brothers, both seen in the first 2 weeks of life. J Am Acad Dermatol 49:132–138. doi:10.1067/mjd.2003.328

    Article  PubMed  Google Scholar 

  54. Rogers M, Greenacre T, Dalla-Pozza L (1987) Neutrophilic dermatosis of myeloproliferative disease in a 10-year-old. Pediatr Dermatol 4:305–312. doi:10.1111/j.1525-1470.1987.tb00798.x

    Article  PubMed  CAS  Google Scholar 

  55. Saxe N, Gordon W (1978) Acute febrile neutrophilic dermatosis (Sweet’s syndrome). Four case reports. S Afr Med J 53:253–256

    PubMed  CAS  Google Scholar 

  56. Schneider DT, Schuppe HC, Schwamborn D, Koerholz D, Lehmann P, Goebel U (1998) Acute febrile neutrophilic dermatosis (Sweet syndrome) as initial presentation in a child with acute myelogenous leukemia. Med Pediatr Oncol 31:178–181. doi:10.1002/(SICI)1096-911X(199809)31:3<178::AID-MPO12>3.0.CO;2-T

    Article  PubMed  CAS  Google Scholar 

  57. Sedel D, Huguet P, Lebbe C, Donadieu J, Odievre M, Labrune P (1994) Sweet syndrome as the presenting manifestation of chronic granulomatous disease in an infant. Pediatr Dermatol 11:237–240. doi:10.1111/j.1525-1470.1994.tb00593.x

    Article  PubMed  CAS  Google Scholar 

  58. Shimizu T, Yoshida I, Eguchi H, Takahashi K, Inada H, Kato H (1996) Sweet syndrome in a child with aplastic anemia receiving recombinant granulocyte colony-stimulating factor. J Pediatr Hematol Oncol 18:282–284. doi:10.1097/00043426-199608000-00009

    Article  PubMed  CAS  Google Scholar 

  59. Sirvent N, Olivier V, Benet L, Maillotte AM, von Bueltzingsloewen A, Mariani R et al (2000) Sweet syndrome in an infant. Arch Pediatr 7:501–504. doi:10.1016/S0929-693X(00)89005-3

    Google Scholar 

  60. Sweet RD (1964) An acute febrile neutrophilic dermatosis. Br J Dermatol 76:349–356. doi:10.1111/j.1365-2133.1964.tb14541.x

    Article  PubMed  CAS  Google Scholar 

  61. Tuerlinckx D, Bodart E, Despontin K, Boutsen Y, Godding V, Ninane J (1999) Sweet’s syndrome with arthritis in an 8-month-old boy. J Rheumatol 26:440–442

    PubMed  CAS  Google Scholar 

  62. Tuncer AM (1988) Acute lymphoblastic leukemia and Sweet’s syndrome. Acta Haematol 80:224

    Article  PubMed  CAS  Google Scholar 

  63. van Kamp H, van den Berg E, Timens W, Kraaijenbrink RA, Halie MR, Daenen SM (1994) Sweet’s syndrome in myeloid malignancy: a report of two cases. Br J Haematol 86:415–417. doi:10.1111/j.1365-2141.1994.tb04757.x

    Article  PubMed  Google Scholar 

  64. von den Driesch P (1994) Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 31:535–560

    Google Scholar 

Download references

Acknowledgments

The authors would like to thank Alexandra Smith for linguistic assistance as well as Joachim Rumpelt, Institute for Pathology, Klinikum Heilbronn, for providing us with the slides of the skin biopsy for the patient in case 2; also Dietmar Ulbricht and Renate Gustorf-Aeckerle, Klinikum Stuttgart, Neuroradiology Department, Katharinenhospital, Stuttgart, Germany for the angiography pictures of the patient in case 2.

Conflicts of interest

All authors declare there are no financial disclosures and no conflict of interest.

Author information

Authors and Affiliations

  1. Department of Pediatrics and Pediatric Rheumatology, Klinikum Stuttgart, Olgahospital, Bismarckstr. 8, 70176, Stuttgart, Germany

    T. Hospach & G. E. Dannecker

  2. Department of Dermatology, Klinikum Stuttgart, Stuttgart, Germany

    P. von den Driesch

Authors
  1. T. Hospach
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. P. von den Driesch
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. G. E. Dannecker
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to T. Hospach.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Hospach, T., von den Driesch, P. & Dannecker, G.E. Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases. Eur J Pediatr 168, 1–9 (2009). https://doi.org/10.1007/s00431-008-0812-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00431-008-0812-0

Keywords

Search

Navigation